Hypokalaemic Alkaloses – RDG

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The Hypokalaemic Alkaloses Rare Disease Group provides expertise in Bartter and Gitelman syndromes and some other very rare conditions. These all affect the kidney’s ability to keep salt and other electrolytes in balance. These conditions are connected by a low concentration of potassium in the blood plasma.

1. The term ‘salt wasting alkaloses’ covers a group of disorders, the majority of which lower blood pressure and in fact lower potassium and/or magnesium. There are several types:

Gitelman syndrome

Types 1 and 2 Bartter syndrome

Type 3 Bartter syndrome

Type 4 Bartter syndrome

EAST syndrome

2. Hypokalaemia with salt retention, in which there is high blood pressure:

Liddle syndrome

They are all rare and need to be diagnosed and managed correctly, as every patient is slightly different in the treatment they need. This particular rare disease group hopes to increase knowledge about these disorders, and set up clinical studies to improve outcome.

Working with Dr Jasmeet Soar (an anaesthetist), Drs Hugh Gallagher and Charlie Tomson have written a clinical guideline for the perioperative management of people with inherited saltwasting alkaloses (Gitelman’s syndrome and Bartter’s syndrome) undergoing non-urgent surgical procedures. It includesrecommendations on pre-operative assessment, minimum acceptable levels of potassium and magnesium and intra- and post- procedural monitoring. The guideline has been endorsed by the Renal Association and the Royal College of Anaesthetists. The aim is to improve the current inconsistent approach whereby patients with saltwasting alkaloses may be denied surgery on the basis of chronic and stable metabolic abnormalities.

The third Gitelman and Bartter Syndrome Patient Information Day was held on Saturday 3rd December 2016 at Resource for London, 356 Holloway Road, London, N7 6PA, from 10.30 – 4.00pm. For further details please click hereor visit the Gitelman Syndrome Online website.

The second Gitelman and Bartter Syndrome Patient Information Day was held on Saturday 13th June 2015 at the Resource for London. A report of the day can be found on the Gitelman Syndrome Online website.

A report of the first Patient Information Day from 2011 can be found here.